External genital abnormalities associated with wilms tumor

Beheshti, Mojtaba; Mancer, J. F. K.; Hardy, Brian; Churchill, Bernard M.; Bailey, John D.

doi:10.1016/0090-4295(84)90413-8

Cited by 14 publications

(4 citation statements)

References 17 publications

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“…Nephropathy associated with Wilms' tumor [5][6][7][8][9][10][11][12][13][14][15][16][17][18] or pseudohermaphroditism [8,14,17,[20][21][22] or both [1][2][3][4]12,14,17,19,20,23] has been described. The coexistence of genital abnormalities, glomerular disease with clinical appearance of nephrotic syndrome and Wilms' tumor constitute a well-defined syndrome: the PWN syndrome or Drash's syndrome named after the author who first suggested that these abnormalities constituted a syn drome [1,2].…”

Section: Discussionmentioning

confidence: 99%

“…The eponym 'Drash's syndrome' has been pro posed for this triad [1,2], The nephropathy has been described as a chronic glomerulonephritis [3,4], Renal involvement has also been reported either in association with Wilms' tumor [5][6][7][8][9][10][11][12][13][14][15][16][17] or with pseudohermaphrodit ism [8,14,[18][19][20], Nephron abnormalities are similar to those described in Drash's syndrome. In all three condi tions the nephropathy, whose onset is usually very preco cious, leads to renal failure.…”

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confidence: 99%

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Nephropathy Associated with Wilms’ Tumor

Gusmano¹,

Perfumo²,

Raspino³

et al. 1987

Nephron

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The association of nephropathy with Wilms’ tumor and male pseudohermaphroditism is known as Drash’s syndrome. The absence of either pseudohermaphroditism or Wilms’ tumor has also been reported. The nephropathy, characterized by proteinuria or nephrotic syndrome, leads rapidly and inevitably to renal failure. The renal findings are those of a chronic glomerulopathy with mesangial hypercellularity, glomerular sclerosis, interstitial infiltration and marked tubular dilation. This infrequent syndrome usually appears in early life, between 1 and 3 years of age. The case of nephropathy associated with Wilms’ tumor we present here is characterized by age and onset: an abrupt onset of renal insufficiency without previous signs of nephropathy in a 13-year-old girl.

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Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

Nephropathy Associated with Wilms’ Tumor

Gusmano¹,

Perfumo²,

Raspino³

et al. 1987

Nephron

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“…The most common extrarenal sites are retroperitoneal, lumbosacral, pelvic, inguinal, female genital organs, and paratesticular [4]. In the early years, Cryptorchidism is frequently known as a congenital de ciency [8]. It has been described that in the differentiation of structures involved in testicular descent, WT1 plays a direct role while genital abnormalities and cryptorchidism are frequently observed in male patients with germline WT1 mutations [8].…”

Section: Discussionmentioning

confidence: 99%

“…In the early years, Cryptorchidism is frequently known as a congenital de ciency [8]. It has been described that in the differentiation of structures involved in testicular descent, WT1 plays a direct role while genital abnormalities and cryptorchidism are frequently observed in male patients with germline WT1 mutations [8]. It is well documented that a tumor suppressor gene for Wilms' tumor is WT1 and accounts for 25% of ERWT cases [3].…”

Section: Discussionmentioning

confidence: 99%

Extrarenal Testicle Wilms Tumor Followed the Operation of Left Hernia Sac High Ligation and Right Cryptorchidism Descent Fixation: A Case Report

Wang

Luo

et al. 2021

Preprint

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Background:The extrarenal Wilms tumor is a rare malignancy in the pediatric population. So far, no extrarenal testicle Wilms tumor followed the operation of left hernia sac high ligation and right cryptorchidism descent fixation has been reported in the literature. Case presentation: We researched an exceptionally unheard case of the extrarenal testicular Wilms tumor after the surgery of left hernia sac high ligation and right cryptorchidism descent fixation in a 2.8 years old boy. The patient was admitted to a hospital due to the evidence of huge scrotum mass six months after the operation of left hernia sac high ligation and right cryptorchidism descent fixation. A huge tumor ascending from the right testicular was found. Conclusions: Histology examination revealed the typical triphasic Wilms tumor elements such as epithelial, mesenchymal, and blastemal areas. This study revealed histopathological, clinical, diagnostic, prognostic, and therapeutic characteristics of this infrequent tumor.

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